Rabbit PTS Polyclonal Antibody | anti-PTS antibody

PTS Polyclonal Antibody

Cat. Num. AAA3011496

• Gene Names: PTS; PTPS
• Reactivity: Human, Mouse, Rat
• Applications: Western Blot, Immunofluorescence
• Purity: The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen and the purity is >95% (by SDS-PAGE).
• Synonyms: PTS; Polyclonal Antibody; PTS Polyclonal Antibody; 6-pyruvoyl tetrahydrobiopterin synthase; PTP synthase; PTPS; anti-PTS antibody

• Host: Rabbit
• Reactivity: Human, Mouse, Rat
• Clonality: Polyclonal
• Isotype: IgG
• Specificity: PTS polyclonal antibody detects endogenous levels of PTS protein.
• Purity/Purification: The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen and the purity is >95% (by SDS-PAGE).
• Form/Format: Rabbit IgG, 1mg/ml in PBS with 0.02% sodium azide, 50% glycerol, pH7.2
• Sequence Length: 145

• Applicable Applications for anti-PTS antibody: Western Blot (WB), Immunofluorescence (IF)
• Application Notes: WB: 1:500-1:2000; IF: 1:50-1:200
• Immunogen: Recombinant full length Human PTS.
• Preparation and Storage: Store at 4 degree C short term.; Aliquot and store at -20 degree C long term.; Avoid freeze-thaw cycles.

Western Blot (WB)

(Western Blot (WB) analysis of PTS polyclonal antibody)

Related Product Information for anti-PTS antibody

The tetrahydrobiopterin (BH4) cofactor is essential for hepatic hydroxylase, which is involved in phenylalanine degradation and catecholamine and serotonin biosynthesis. BH4 is also an essential and limiting cofactor for all types of nitric oxide synthase. BH4 deficiency results in hyperphenylalaninemia and monoamine neurotransmitter depletion and is most commonly due to autosomal recessive mutation in 6-pyruvoyltetrahydropterin synthase (PTPS), the second enzyme for BH4 biosynthesis. The active site of PTPS consists of the pterin-anchoring Glu A107 neighbored by two catalytic motifs: a Zn(II) binding site and an inter-subunit catalytic triad formed by Cys A42, Asp B88 and His B89. The active site of PTPS undergoes a Zn and Mg-dependent reaction that includes a triphosphate elimination, a stereospecific reduction and the oxidation of both side hydroxyl groups. The catalytic triad of PTPS is involved in the deprotonation of the side-chain carbons of substrates. In addition, Ser 19 of human PTPS may be a substrate for cGMP-dependent protein kinase type II phosphorylation in vivo, which is essential for normal activity of PTPS.

NCBI and Uniprot Product Information

• NCBI GI #: 4506331
• NCBI GeneID: 5805
• NCBI Accession #: NP_000308.1
• NCBI GenBank Nucleotide #: NP_000308.1
• UniProt Accession #: Q03393
• Molecular Weight: ~ 16kDa
• NCBI Official Full Name: 6-pyruvoyl tetrahydrobiopterin synthase
• NCBI Official Synonym Full Names: 6-pyruvoyltetrahydropterin synthase
• NCBI Official Symbol: PTS
• NCBI Official Synonym Symbols: PTPS
• NCBI Protein Information: 6-pyruvoyl tetrahydrobiopterin synthase
• UniProt Protein Name: 6-pyruvoyl tetrahydrobiopterin synthase
• Protein Family: PTS system EIIBC component
• UniProt Gene Name: PTS
• UniProt Synonym Gene Names: PTP synthase; PTPS
• UniProt Entry Name: PTPS_HUMAN

NCBI Description

The enzyme encoded by this gene catalyzes the elimination of inorganic triphosphate from dihydroneopterin triphosphate, which is the second and irreversible step in the biosynthesis of tetrahydrobiopterin from GTP. Tetrahydrobiopterin, also known as BH(4), is an essential cofactor and regulator of various enzyme activities, including enzymes involved in serotonin biosynthesis and NO synthase activity. Mutations in this gene result in hyperphenylalaninemia. [provided by RefSeq, Oct 2008]

Uniprot Description

PTS: Involved in the biosynthesis of tetrahydrobiopterin, an essential cofactor of aromatic amino acid hydroxylases. Catalyzes the transformation of 7,8-dihydroneopterin triphosphate into 6- pyruvoyl tetrahydropterin. Defects in PTS are the cause of BH4-deficient hyperphenylalaninemia type A (HPABH4A); also called 6-pyruvoyl-tetrahydropterin synthase deficiency (PTS deficiency) or hyperphenylalaninemia tetrahydrobiopterin-deficient due to PTS deficiency. HPABH4A is an autosomal recessive disorder characterized by depletion of the neurotransmitters dopamine and serotonin, and clinically by severe neurological symptoms unresponsive to the classic phenylalanine-low diet. Belongs to the PTPS family.

Protein type: Lyase; Mitochondrial; Cofactor and Vitamin Metabolism - folate biosynthesis; EC 4.2.3.12

Chromosomal Location of Human Ortholog: 11q22.3

Cellular Component: mitochondrion; cytoplasm; cytosol

Molecular Function: identical protein binding; protein homodimerization activity; metal ion binding; 6-pyruvoyltetrahydropterin synthase activity

Biological Process: amino acid metabolic process; tetrahydrobiopterin biosynthetic process; central nervous system development; regulation of nitric-oxide synthase activity; nitric oxide metabolic process

Disease: Hyperphenylalaninemia, Bh4-deficient, A